Singh S P, Besner G E, Schauer G M
Department of Pathology, Ohio State University Medical Center, Columbus, USA.
Pediatr Pathol Lab Med. 1997 Nov-Dec;17(6):951-8.
This report describes the clinical and histologic features of a pulmonary tumor in a 14-year-old girl that is most consistent with a rare entity described in the literature as "pulmonary endodermal tumor resembling fetal lung" (PET). This tumor is composed of glycogen-rich columnar cells forming complex glands with focal festooning and mitotic activity, admixed with solid "morules" of cells with eosinophilic cytoplasm and focal nuclear clearing. Patchy tumor necrosis and a bland stroma were also present. Immunoreactivity for carcinoembryonic antigen (CEA), alpha 1-antichymotrypsin, and 12E7 was present in glandular cells and for human chorionic gondatropin (HCG), alpha 1-antichymotrypsin, and 12E7 in morular cells. Ultrastructural features are those of an epithelial tumor. Related entities have been called "pulmonary blastoma lacking sarcomatous elements" and "adenocarcinoma of fetal lung type." Most cases of PET have occurred in adults, and the histologic features thought to have prognostic significance in small published series are applied to our case, in which the patient remains well and without evidence of tumor recurrence or metastasis for 28 months following local resection as the sole treatment.
本报告描述了一名14岁女孩肺部肿瘤的临床和组织学特征，该肿瘤与文献中描述的一种罕见实体“类似胎儿肺的肺内胚层肿瘤”（PET）最为相符。此肿瘤由富含糖原的柱状细胞组成，形成具有局灶性叶状皱襞和有丝分裂活性的复杂腺体，混有嗜酸性细胞质和局灶性核空晕的实性“桑椹胚”细胞。还存在散在的肿瘤坏死和淡染的间质。腺细胞中癌胚抗原（CEA）、α1抗胰凝乳蛋白酶和12E7呈免疫反应阳性，桑椹胚细胞中人绒毛膜促性腺激素（HCG）、α1抗胰凝乳蛋白酶和12E7呈免疫反应阳性。超微结构特征为上皮性肿瘤。相关实体曾被称为“无肉瘤成分的肺母细胞瘤”和“胎儿肺型腺癌”。PET的大多数病例发生在成年人中，在已发表的少量病例系列中被认为具有预后意义的组织学特征应用于我们的病例，该患者在作为唯一治疗的局部切除术后28个月情况良好，无肿瘤复发或转移迹象。
